Panayiotopoulos syndrome
The significant increase in plasma vasopressin may explain the emetic autonomic symptoms. Misdiagnosis The distinctive clinical features particularly lengthy seizures and ictus emeticus means that the diagnosis of Panayiotopoulos syndrome is easy. However, these are frequently mistaken as nonepileptic conditions such as acute encephalitis, syncope, migraine, cyclic vomiting syndrome, motion sickness, sleep disorder, or gastroenteritis.Covanis A. Panayiotopoulos Syndrome: A Benign Childhood Autonomic Epilepsy Frequently Imitating Encephalitis, Syncope, Migraine, Sleep Disorder, or Gastroenteritis. Pediatrics 2006; 118(4):e1237-e1243 . The consequence is avoidable misdiagnosis, high morbidity, and costly mismanagement. Autonomic seizures and autonomic status epilepticus as occur in Panayiotopoulos syndrome have not been described in other epileptic syndromes in that sequence though 10–20 per cent of children with the same seizure semiology may have cerebral pathology. The major problem is to recognize emetic and other autonomic manifestations as seizure events and not to dismiss them or erroneously to consider them as unrelated to the ictus and a feature of encephalitis, migraine, syncope or gastro-enteritis. Prognosis Panayiotopoulos syndrome is remarkably benign in terms of its evolution.Panayiotopoulos CP. Panayiotopoulos syndrome: a common and benign childhood epileptic syndrome. London: John Libbey & Company; 2002.Koutroumanidis M. Panayiotopoulos Syndrome: An Important Electroclinical Example of Benign Childhood System Epilepsy. Epilepsia 2007; 48(6):1044-1053Caraballo R, Cersosimo R, Fejerman N. Panayiotopoulos syndrome: a prospective study of 192 patients. Epilepsia 2007; 48(6):1054-1061.Panayiotopoulos CP, Michael M, Sanders S, Valeta T, Koutroumanidis M. Benign childhood focal epilepsies: assessment of established and newly recognized syndromes. Brain 2008; 131(Pt 9):2264-2286.Specchio N, Trivisano M, Balestri M, Cappelletti S, Di Ciommo V, Gentile S et al. Panayiotopoulos syndrome: A Clinical, EEG and Neuropsychological Study of 93 Consecutive Patients. Epilepsia 2010; 51(10):2098-2107. The risk of developing epilepsy in adult life is probably no more than of the general population. Most patients have one or 2-5 seizures. Only a third of patients may have more than 5 seizures, and these may be frequent, but outcome is again favorable. However, one fifth of patients may develop other types of infrequent, usually rolandic seizures during childhood and early teens. These are also age-related and remit before the age of 16 years. Atypical evolutions with absences and drop attacks are exceptional. Children with pre-existing neurobehavioral disorders tend to be pharmacoresistant and have frequent seizures though these also remit with age. Formal neuropsychological assessment of children with Panayiotopoulos syndrome showed that these children have normal IQ and they are not on any significant risk of developing cognitive and behavioural aberrations, which when they occur they are usually mild reversible.Specchio N, Trivisano M, Balestri M, Cappelletti S, Di Ciommo V, Gentile S et al. Panayiotopoulos syndrome: A Clinical, EEG and Neuropsychological Study of 93 Consecutive Patients. Epilepsia 2010; 51(10):2098-2107. Prognosis of cognitive function is good even for patients with atypical evolutions.Caraballo R, Cersosimo R, Fejerman N. Panayiotopoulos syndrome: a prospective study of 192 patients. Epilepsia 2007; 48(6):1054-1061. However, though Panayiotopoulos syndrome is benign in terms of its evolution, autonomic seizures are potentially life threatening in the rare context of cardiorespiratory arrest.Ferrie CD, Caraballo R, Covanis A, Demirbilek V, Dervent A, Fejerman N et al. Autonomic status epilepticus in Panayiotopoulos syndrome and other childhood and adult epilepsies: a consensus view. Epilepsia 2007; 48(6):1165-1172. Management Continuous prophylactic antiepileptic drug (AED) treatment may not be needed particularly for children with only 1-2 or brief seizures. This is probably best reserved for children whose seizures are unusually frequent, prolonged, distressing, or otherwise significantly interfering with the child’s life. There is no evidence of superiority of monotherapy with any particular common AED.Ferrie C, Caraballo R, Covanis A, Demirbilek V, Dervent A, Kivity S et al. Panayiotopoulos syndrome: a consensus view. Dev Med Child Neurol 2006; 48(3):236-240.Panayiotopoulos CP, Michael M, Sanders S, Valeta T, Koutroumanidis M. Benign childhood focal epilepsies: assessment of established and newly recognized syndromes. Brain 2008; 131(Pt 9):2264-2286. Autonomic status epilepticus in the acute stage needs thorough evaluation for proper diagnosis and assessment of the neurologic/autonomic state of the child. "Rescue" benzodiazepines are commonly used to terminate it. Aggressive treatment should be avoided because of the risk of iatrogenic complications, including cardiovascular arrest. There is some concern that intravenous lorazepam and/or diazepam may precipitate cardiovascular arrest. Lacroix L, Fluss J, Gervaix A, Korff CM. Benzodiazepines in the acute management of seizures with autonomic manifestations: anticipate complications! Epilepsia 2011 October;52(10):e156-e159. Early parental treatment is more effective than late emergency treatment. Buccal midazolam is probably the first choice medication for out of hospital termination of autonomic status epilepticus which should be administered as soon as the child shows evidence of onset of its habitual autonomic seizures. Parental education about Panayiotopoulos syndrome is the cornerstone of correct management. The traumatizing, sometimes long-lasting effect on parents is significant particularly because autonomic seizures may last for many hours compounded by physicians’ uncertainty regarding diagnosis, management, and prognosis.Valeta T. Parental attitude, reaction and education in benign childhood focal seizures. In: Panayiotopoulos CP, editor. The Epilepsies: Seizures, Syndromes and Management. Oxford: Bladon Medical Publishing; 2005. 258-261. References Category:Epilepsy types Category:Syndromes